Aplasia Cutis Congenita- Information, Treatment & Prevention

By: Juliet Cohen

Aplasia cutis congenita is region of a heterogenous group of disorders. The reason is not sure as it is the outcome of much than one disease procedure. Genetic factors, teratogens, compromised vasculature to the rind, and injury are all implicated. Exogenic causes (amniotic attachment, intra uterine injury or vascular abnormalities) have been accused but numerous genetic cases with improper autosomal predominant infection have been reported. New born with thoracic dysplasia associated with lung dysplasia usually die during the neonatal period due to respiratory failure. In those who survive, chronic renal failure is a common cause of death. If aplasia cutis congenita occurs on the scalp, there may be a defect of the skull underneath the missing patch. In this case, there is often distorted hair growth around the patch, known as the hair collar sign.

Aplasia cutis congenita may be circular, elliptical, linear, or stellate in shape. At birth, the lesions may get already healed with scarring or may stay superficially eroded to profoundly ulcerated, occasionally involving the dura or the meninges. Aplasia cutis congenita is almost frequently a harmless isolated flaw, but it can be associated with new physiological anomalies or deformity syndromes. Larger lesions associated with underlying bony defects may result in death secondary to central nervous system infection or hemorrhage from the sagittal sinus. The affected area is typically covered with a thin, transparent membrane. The skull and underlying areas may be visible and be abnormally developed. Aplasia cutis congenita may be the primary disorder or it may occur in association with other underlying disorders.

Aplasia cutis congenita is characterized by the absence of a part of rind in a localized or widespread region at birth. It almost usually manifests as a lonely flaw on the scalp, but sometimes it may happen as dual lesions. Defects in the rind that organize early in gestation may mend before saving and seem as an atrophic, membranous, or parchmentlike scratch with associated alopecia, whereas little old defects existing as ulcerations. Most lesions happen on the scalp vertex just lateral to the midline, but defects may too happen on the cheek, the body, or the limbs, sometimes symmetrically. The lesions are noninflammatory and well demarcated. The appearance of the lesions varies, depending on when they occur during intrauterine development. Lesions that form early in gestation may heal before delivery and appear as an atrophic, membranous, parchmentlike or fibrotic alopecic scar.

Usually the alone handling required for aplasia cutis congenital is mild cleansing of the stricken region and the application of a silver sulfadiazine or new salve to forbid the piece from drying away. Other treatment is rarely necessary because the erosions and the ulcerations almost always heal spontaneously. Surgical repair is not usually indicated if the defect is small. Recovery is uneventful, with gradual epithelialization and formation of a hairless, atrophic scar over several weeks. Small underlying bony defects usually close spontaneously during the first year of life. Truncal and limb defects, despite their large size, usually epithelialize and form atrophic scars, which can later be revised if necessary. Aplasia cutis congenita of the scalp may be complicated by sagittal sinus hemorrhage or thrombosis, and primary closure with scalp flaps may prevent a potentially fatal outcome.

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